Skip to main content

Health Encyclopedia

Search the Health Encyclopedia

Urticaria pigmentosa

Definition

Urticaria pigmentosa is a skin disease that produces patches of darker skin and very bad itching. Hives can develop when these skin areas are rubbed.

Alternative Names

Mastocytosis; Mastocytoma

Causes

Urticaria pigmentosa occurs when there are too many inflammatory cells (mast cells) in the skin. Mast cells are immune system cells that help the body fight infections. Mast cells make and release histamine, which causes nearby tissues to become swollen and inflamed.

Things that can trigger histamine release and skin symptoms include:

  • Rubbing the skin
  • Infections
  • Exercise
  • Drinking hot liquids, eating spicy food
  • Sunlight, exposure to cold
  • Drugs, such as aspirin or other NSAIDs, codeine, morphine, x-ray dye, some anesthesia drugs, alcohol

Urticaria pigmentosa is most common in children. It can also occur in adults.

Symptoms

The main symptom is brownish patches on the skin. These patches contain histamine. When histamine is triggered, the patches develop into hive-like bumps. Younger children may develop a blister that is filled with fluid if the bump is scratched.

The face may also get red quickly.

In severe cases, these symptoms may occur:

  • Diarrhea
  • Fainting (uncommon)
  • Headache
  • Rapid heartbeat

Exams and Tests

The health care provider will examine the skin. The provider may suspect urticarial pigmentosa when the skin patches are rubbed and raised bumps (hives) develop. This is called the Darier sign.

Tests to check for this condition are:

  • Skin biopsy to look for a higher number of mast cells
  • Urine histamine
  • Blood tests for blood cell counts and blood tryptase levels (tryptase is an enzyme found in mast cells)

Treatment

Antihistamine medicines can help relieve symptoms such as itching and flushing. Talk to your provider about which type of antihistamine to use. Corticosteroids applied on the skin and light therapy can also be used in some cases.

Your provider may prescribe other kinds of medicine to treat symptoms of severe and unusual forms of urticaria pigmentosa.

Outlook (Prognosis)

Urticaria pigmentosa goes away by puberty in about half of affected children. Symptoms usually get better in others as they grow into adulthood.

In adults, urticaria pigmentosa can lead to systemic mastocytosis. This is a serious condition that can affect bones, the brain, nerves, and the digestive system.

Possible Complications

The main problems are discomfort from itching and concern about the appearance of the spots. Other problems such as diarrhea and fainting are rare.

Bee stings may also cause a bad allergic reaction in people with urticaria pigmentosa. Ask your provider if you should carry an epinephrine kit to use if you get a bee sting.

When to Contact a Medical Professional

Call your provider if you notice symptoms of urticaria pigmentosa.

References

Habif TP. Urticaria, angioedema, and pruritus. In: Habif TP, ed. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 6th ed. Philadelphia, PA: Elsevier; 2016:chap 6.

Paller AS, Mancini AJ. Cutaneous tumors and tumor syndromes. In: Paller AS, Mancini AJ, eds. Hurwitz Clinical Pediatric Dermatology. 5th ed. Philadelphia, PA: Elsevier; 2016:chap 9.

Review Date:10/31/2016
Reviewed By:Kevin Berman, MD, PhD, Atlanta Center for Dermatologic Disease, Atlanta, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997-A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

adam.com

The Agency for Health Care Administration (Agency) and this website do not claim the information on, or referred to by, this site is error free. This site may include links to websites of other government agencies or private groups. Our Agency and this website do not control such sites and are not responsible for their content. Reference to or links to any other group, product, service, or information does not mean our Agency or this website approves of that group, product, service, or information.

Additionally, while health information provided through this website may be a valuable resource for the public, it is not designed to offer medical advice. Talk with your doctor about medical care questions you may have.

Health
Outcome Data

No data available for this condition/procedure.

Read More

Bee poison

Fainting *

Itching

Pulse - bounding

Rashes

Vesicles


* Has Related Health Outcome Information

Health Encyclopedia

More Features

We Appreciate Your Feedback!
1. Did you find this information useful?
         Yes
         No
2. Would you recommend this website to family and friends?
         Yes
         No